GRFoma

GRFomas, first described in 1982,66,67 are neuroendocrine tumors that secrete excessive amounts of GRF. Patients with these tumors have acromegaly. GRF is a peptide that is similar to VIP.66'67 GRFomas can occur (in order of decreasing frequency) in the lung (bronchus), pancreas, jejunum, adrenal, and retroperitoneum.68 Patients with GRFoma commonly have a large pancreatic neuroendocrine tumor (>6 cm) that is metastatic in one third of cases at diagnosis. Approximately 50% of patients with GRFomas also have Zollinger-Ellison syndrome and 33% have MEN 1 (Table 84-6).

GRFoma is anticipated when a patient has acromegaly and a pancreatic mass. Liver metastases and peptic ulcer disease should also be considered.66 68 The diagnosis can be confirmed by performing a plasma assay for GRF and a CT scan of the abdomen to identify a pancreatic or liver tumor. Octreotide therapy can relieve the signs and symptoms of acromegaly. Surgical resection should be attempted in these patients because complete resection may be curative, and debulking may decrease symptoms and prolong survival.

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